Primary Hepatic Carcinoid Tumor (PHCT). A Rare Entity. Review of the Literature

Abstract

Carcinoid tumors are rare, but are the most common gastrointestinal (GI) neuroendocrine tumors. Within the GI tract, most carcinoids arise in the small intestine (45%, most commonly in the ileum), followed by rectum (20%), appendix (16%), colon (11%), and stomach (7%). PHCT is a very rare entity. Its description has been limited to case reports. We describe a case of PHCT and a review of the literature. Case: 49 year old Caucasian female with history of IBS and a negative abdominal CT 4 years earlier presents with abdominal pain, nausea and vomiting. Repeat imaging showed an 8.7 cm by 6.7 cm mass in the left lobe of the liver with a necrotic component. CT guided FNA was positive for Carcinoid Tumor. Extensive workup for a primary site, including an EGD, colonoscopy, UGI SBFT, and an octreotide scan were all negative. 24-hour urine 5 HIAA was normal. Serum serotonin was low. Patient underwent left hepatic lobectomy with clear margin of resection. Immunohistochemical staining showed strong reactivity to neoplastic cells for cytokeratin (AE1/3) and synaptophysin, and light to medium cytoplasmic staining for chromogranin. There was no reactivity for Hep-par-1 and CEA. There was nuclear staining for CDX-2. Repeat CT abdomen 2 years later shows no evidence of recurrent tumor. Discussion: In 2001, Iwao et al from Japan described a case report of PHCT and reviewed 53 previously published case reports. Analysis of these published cases indicates that PHCT occurs in the middle age (mean age = 48.2 years) and is more frequent in females (males/females = 20/33 cases). Of the symptomatic patients, the major findings were abdominal pain, fullness, and/or a palpable mass (56% of symptomatic patients). In contrast, only 2 cases out of 53 presented with symptoms of typical carcinoid syndrome. Surgical resection is the treatment primarily recommended with an 18% of recurrence rate and a 74% of a survival rate after 5 years. Treatment of inoperable cases has varied. Bastaki et at described percutaneous embolization of the tumor followed by complete dearterializations of the lesion. Knox et at performed a survival analysis with the Kaplan-Meier method on 48 published cases cited in the English literature. Actuarial 5- and 10-year survival for all patients was 78% and 59%, respectively, whereas for resected patients, 10-year survival was 68%. PHCT is a rare disorder. Diagnosis can be made by immunohistochemical staining and appropriate workup to exclude other primary sites. Treatment is surgical resection if technically feasible.